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Childhood hearing loss

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https://www.readbyqxmd.com/read/27924730/congenital-malformations-attributed-to-prenatal-exposure-to-cyclophosphamide
#1
Padmanabhan Rengasamy
Cyclophosphamide (CPA) remains one of the most widely prescribed anticancer drugs. It is also used in the treatment of rheumatoid arthritis, childhood nephrotic syndrome and systemic lupus erythematosus. It is a potent immunosuppressive agent. It is commonly used in blood and bone marrow transplantation. With the growing trend among women postponing childbearing, the number of women who are diagnosed with breast cancer is also increasing thus escalating the chances of exposure of the unborn child to antineoplastic drugs...
December 6, 2016: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/27918236/meniere-s-migraine-and-motion-sickness
#2
John F Golding, Mitesh Patel
CONCLUSION: Elevated Motion Sickness Susceptibility (MSS) in Meniere?s disease (MD) is likely to be a consequence of the onset of MD and not migraine per se. OBJECTIVES: Pathologies of the vestibular system influence MSS. Bilateral vestibular deficits lower MSS, vestibular neuritis or benign paroxysmal positional vertigo have little overall effect, whereas vestibular migraine elevates MSS. However, less is known about MSS in MD, a condition in which many patients experience vestibular loss and migraine symptoms...
December 5, 2016: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/27896131/case-report-5%C3%A2-year-follow-up-of-adult-late-onset-mitochondrial-encephalomyopathy-with-lactic-acid-and-stroke-like-episodes-melas
#3
Kiri Sunde, Patrick R Blackburn, Anvir Cheema, Jennifer Gass, Jessica Jackson, Sarah Macklin, Paldeep S Atwal
Mitochondrial encephalomyopathy with lactic acid and stroke-like episodes (MELAS) is a multisystem mitochondrial disorder that typically presents in childhood. We describe the follow-up of a patient who was diagnosed with late-onset MELAS at the age of 49. Her clinical course includes sensorineural hearing loss, seizures, and multiple episodes of stroke-like metabolic crises. Molecular genetic testing on whole blood revealed 31% heteroplasmy of a m.3243A > G variant in the mtDNA, the causative variant in approximately 80% of MELAS cases...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27776808/evaluation-of-family-history-of-permanent-hearing-loss-in-childhood-as-a-risk-indicator-in-universal-screening
#4
Mercedes Valido Quintana, Ángeles Oviedo Santos, Silvia Borkoski Barreiro, Alfredo Santana Rodríguez, Ángel Ramos Macías
INTRODUCTION AND OBJECTIVE: Sixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation. MATERIAL AND METHOD: This was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response...
October 21, 2016: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/27771768/advances-in-genetic-hearing-loss-cib2-gene
#5
Agnieszka Jacoszek, Agnieszka Pollak, Rafał Płoski, Monika Ołdak
Hearing plays a crucial role in human development. Receiving and processing sounds are essential for the advancement of the speech ability during the early childhood and for a proper functioning in the society. Hearing loss is one of the most frequent disabilities that affect human senses. It can be caused by genetic or environmental factors or both of them. Calcium- and integrin-binding protein 2 (CIB2) is one of the recently identified genes, involved in HI pathogenesis. CIB2 is widely expressed in various human and animal tissues, mainly in skeletal muscle, nervous tissue, inner ear, and retina...
October 22, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27761875/saving-deaf-children-screening-for-hearing-loss-as-a-public-interest-case
#6
Sigrid Bosteels, Michel Vandenbroeck, Geert Van Hove
New-born screening programs for congenital disorders and chronic disease are expanding worldwide and children "at risk" are identified by nationwide tracking systems at the earliest possible stage. These practices are never neutral and raise important social and ethical questions. An emergent concern is that a reflexive professionalism should interrogate the ever earlier interference in children's lives. The Flemish community of Belgium was among the first to generalize the screening for hearing loss in young children and is an interesting case to study the public justification of early interventions for families with deaf children...
October 19, 2016: Journal of Bioethical Inquiry
https://www.readbyqxmd.com/read/27746216/early-age-noise-exposure-increases-loudness-perception-a-novel-animal-model-of-hyperacusis
#7
Ana'am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 h) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies...
October 13, 2016: Hearing Research
https://www.readbyqxmd.com/read/27729161/associations-between-childhood-hearing-loss-and-behavioural-and-academic-difficulties-a-danish-cohort-study
#8
Janni Niclasen, Carsten Obel, Christian Guldager, Simone Pleinert, Jesper Dammeyer
OBJECTIVE: Negative associations between hearing loss (HL) and behavioural and academic difficulties have been reported. However, most studies are based on small clinical samples. The aim of the present study was to investigate such associations using data from a large-scale non-clinical Danish birth cohort controlling for a large number of relevant confounding factors. METHODS: The study applied data from the Aarhus Birth Cohort's 10-12-year-old follow-up (N = 7599)...
November 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27729120/younger-patients-with-come-are-more-likely-to-have-mucoid-middle-ear-fluid-containing-mucin-muc5b
#9
Vanessa Duah, Zhen Huang, Stephanie Val, Christie DeMason, Marain Poley, Diego Preciado
BACKGROUND: Chronic otitis media (COM) is one of the most common childhood diseases. Its pathophysiology is complex and multifactorial. The role of specific mucin glycoprotein subtypes in OM is only recently being elucidated. OBJECTIVE: To determine the relationship between middle ear fluid mucins and clinical variables of patients needing tympanostomy tubes (TT). METHODS: Middle ear effusions (MEE) from children receiving TT were collected over a 2-year period...
November 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27726050/mosaicism-in-atp1a3-related-disorders-not-just-a-theoretical-risk
#10
Marie Hully, Juliette Ropars, Laurence Hubert, Nathalie Boddaert, Marlene Rio, Mathieu Bernardelli, Isabelle Desguerre, Valerie Cormier-Daire, Arnold Munnich, Pascale de Lonlay, Louise Reilly, Claude Besmond, Nadia Bahi-Buisson
Mutations in ATP1A3 are involved in a large spectrum of neurological disorders, including rapid onset dystonia parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS), with recent descriptions of overlapping phenotypes. In AHC, a few familial cases of autosomal dominant inheritance have been reported, along with cases of de novo sporadic mutations. In contrast, autosomal dominant inheritance has frequently been associated with RDP and CAPOS...
October 10, 2016: Neurogenetics
https://www.readbyqxmd.com/read/27723885/cost-effectiveness-of-universal-and-targeted-newborn-screening-for-congenital-cytomegalovirus-infection
#11
Soren Gantt, Francois Dionne, Fred K Kozak, Oran Goshen, David M Goldfarb, Albert H Park, Suresh B Boppana, Karen Fowler
Importance: Congenital cytomegalovirus (cCMV) infection is a major cause of childhood deafness. Most cCMV infections are not diagnosed without newborn screening, resulting in missed opportunities for directed care. Objective: To estimate the cost-effectiveness of universal and targeted newborn cCMV screening programs compared with no cCMV screening. Design, Setting, and Participants: Models were constructed using rates and outcomes from prospective cohort studies of newborn cCMV screening in US postpartum care and early hearing programs...
December 1, 2016: JAMA Pediatrics
https://www.readbyqxmd.com/read/27707755/constitutive-activation-of-dia1-diaph1-via-c-terminal-truncation-causes-human-sensorineural-hearing-loss
#12
Takehiko Ueyama, Yuzuru Ninoyu, Shin-Ya Nishio, Takushi Miyoshi, Hiroko Torii, Koji Nishimura, Kazuma Sugahara, Hideaki Sakata, Dean Thumkeo, Hirofumi Sakaguchi, Naoki Watanabe, Shin-Ichi Usami, Naoaki Saito, Shin-Ichiro Kitajiri
DIAPH1 encodes human DIA1, a formin protein that elongates unbranched actin. The c.3634+1G>T DIAPH1 mutation causes autosomal dominant nonsyndromic sensorineural hearing loss, DFNA1, characterized by progressive deafness starting in childhood. The mutation occurs near the C-terminus of the diaphanous autoregulatory domain (DAD) of DIA1, which interacts with its N-terminal diaphanous inhibitory domain (DID), and may engender constitutive activation of DIA1. However, the underlying pathogenesis that causes DFNA1 is unclear...
November 2, 2016: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/27688594/the-p1-biomarker-for-assessing-cortical-maturation-in-pediatric-hearing-loss-a-review
#13
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
https://www.readbyqxmd.com/read/27680221/a-case-of-cerebrotendinous-xanthomatosis-mimicking-the-clinical-phenotype-of-mitochondrial-disease-with-a-novel-frame-shift-mutation-c-43_44-delgg-in-cyp27a1-gene-exon-1
#14
Junpei Koge, Shintaro Hayashi, Hiroo Yamaguchi, Takahisa Tateishi, Hiroyuki Murai, Jun-Ichi Kira
A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27650356/long-term-auditory-complications-after-childhood-cancer-a-report-from-the-swiss-childhood-cancer-survivor-study
#15
Annette Weiss, Grit Sommer, Rahel Kasteler, Katrin Scheinemann, Michael Grotzer, Martin Kompis, Claudia E Kuehni
BACKGROUND: Auditory complications are an adverse event of childhood cancer treatment, especially common in children treated with platinum chemotherapy or cranial radiation. Variation between diagnostic childhood cancer groups has rarely been studied, and we do not know if the burden of auditory complications has changed over the last decades. PROCEDURE: Within the Swiss Childhood Cancer Survivor Study, we sent a questionnaire to all survivors who were diagnosed at age 16 years or less between 1976 and 2005...
September 21, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27648330/abnormal-congenital-location-of-stapes-superstructure-clinical-and-embryological-implications
#16
Vânia Henriques, Rafaela Teles, Ana Sousa, Roberto Estevão, Jorge Rodrigues, Alexandra Gomes, Francisco Silva, Ângelo Fernandes, Fausto Fernandes
Congenital middle ear malformations are rare. Most part of them are usually associated with other malformations, such as aural atresia, microtia, and dysmorphic craniofacial features. A clinical case of a 24-year-old male with a right-sided conductive hearing loss since his childhood, without craniofacial malformation, is presented. He was proposed for exploratory tympanotomy under the suspicious diagnosis of otosclerosis. The surgery revealed an abnormal location of stapes' superstructure, which was attached to the promontory and had an isolated and mobile osseous footplate in the oval window...
2016: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/27636391/incidence-of-pediatric-superior-semicircular-canal-dehiscence-and-inner-ear-anomalies-a-large-multicenter-review
#17
Eric M Sugihara, Seilesh C Babu, Dennis J Kitsko, Michael S Haupert, Prasad J Thottam
OBJECTIVE: To determine the pediatric incidence and association of superior semicircular canal dehiscence (SSCD) with inner ear (IE) anomalies. STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary referral centers. PATIENTS: Children less than 18 years who received a 0.5 mm or less collimated computed tomography study including the temporal bones between 2010 and 2013 for reasons including, but not limited to, hearing loss, trauma, and infection...
October 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27634470/de-novo-p-arg756cys-mutation-of-atp1a3-causes-an-atypical-form-of-alternating-hemiplegia-of-childhood-with-prolonged-paralysis-and-choreoathetosis
#18
Hikaru Kanemasa, Ryoko Fukai, Yasunari Sakai, Michiko Torio, Noriko Miyake, Sooyoung Lee, Hiroaki Ono, Satoshi Akamine, Kei Nishiyama, Masafumi Sanefuji, Yoshito Ishizaki, Hiroyuki Torisu, Hirotomo Saitsu, Naomichi Matsumoto, Toshiro Hara
BACKGROUND: Alternating hemiplegia of childhood (AHC) is a rare neurological disorder that manifests recurrent attacks of hemiplegia, oculogyric, and choreoathetotic involuntary movements. De novo mutations in ATP1A3 cause three types of neurological diseases: AHC; rapid-onset dystonia-Parkinsonism (RDP); and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndromes. It remains to be determined whether or not a rare mutation in ATP1A3 may cause atypical phenotypes...
September 15, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27620717/delayed-diagnosis-of-pendred-syndrome
#19
Natalie Smith, Jean-Marie U-King-Im, Janaka Karalliedde
We describe a case of a delayed diagnosis of Pendred syndrome. The patient had a history of hearing loss from childhood and presented to the endocrine clinic when aged 32 with an enlarging goitre. The characteristic Mondini deformity was noted on auditory canal MRI scan. Genetic tests confirmed the diagnosis. Pendred syndrome is an autosomal recessive disorder, characterised by congenital sensorineural hearing loss, goitre and impaired iodide organification and can present at any age. We highlight to the general physician the classical features of this syndrome that would aid early diagnosis...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27613397/cross-modal-plasticity-in-developmental-and-age-related-hearing-loss-clinical-implications
#20
Hannah Glick, Anu Sharma
This review explores cross-modal cortical plasticity as a result of auditory deprivation in populations with hearing loss across the age spectrum, from development to adulthood. Cross-modal plasticity refers to the phenomenon when deprivation in one sensory modality (e.g. the auditory modality as in deafness or hearing loss) results in the recruitment of cortical resources of the deprived modality by intact sensory modalities (e.g. visual or somatosensory systems). We discuss recruitment of auditory cortical resources for visual and somatosensory processing in deafness and in lesser degrees of hearing loss...
September 6, 2016: Hearing Research
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